Phenotypes associated with this allele

• Allele Symbol: Bicc1^jcpk
• Allele Name: juvenile congenital polycystic kidney disease
• Allele ID: MGI:1856920
• Summary: 5 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Bicc1^jcpk/Bicc1^jcpk	involves: 101 * C3H * C57BL/6J * T STOCK	MGI:3582933
hm2	Bicc1^jcpk/Bicc1^jcpk	involves: 101 * C3H * T STOCK	MGI:3582952
ht3	Bicc1^jcpk/Bicc1^+	involves: 101 * C3H * T STOCK	MGI:3582953
ht4	Bicc1^jcpk/Bicc1^jcpk-bpk	involves: 101 * BALB/c * C3H * C57BL/6J * T STOCK	MGI:3582934
cx5	Bicc1^jcpk/T(2;10)67Gso	involves: 101 * C3H * C3H/Rl * C57BL/6 * C57BL/RlGso * SEC/RlGso	MGI:3583140

Genotype
MGI:3582933

hm1

• Allelic Composition: Bicc1^jcpk/Bicc1^jcpk
• Genetic Background: involves: 101 * C3H * C57BL/6J * T STOCK

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

renal/urinary system

kidney cortex cyst ( J:36348 )

• cysts in the proximal and distal tubules

increased glomerular capsule space ( J:36348 )

• dilation of Bowman's space in 95% of glomeruli

liver/biliary system

abnormal bile duct morphology ( J:36348 )

• branched bile ductules are lined with hyperplastic epithelium

abnormal portal triad morphology ( J:36348 )

• the portal triad is expanded by hematopoietic cells and immature stromal cells

endocrine/exocrine glands

abnormal bile duct morphology ( J:36348 )

• branched bile ductules are lined with hyperplastic epithelium

growth/size/body

kidney cortex cyst ( J:36348 )

• cysts in the proximal and distal tubules

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
autosomal recessive polycystic kidney disease		DOID:0110861	OMIM:263200	J:36348

Genotype
MGI:3582952

hm2

• Allelic Composition: Bicc1^jcpk/Bicc1^jcpk
• Genetic Background: involves: 101 * C3H * T STOCK

mortality/aging

postnatal lethality, complete penetrance ( J:23047 , J:27506 )

• generally die between 7 to 10 days after birth with none surviving longer than 20 days (J:27506)

renal/urinary system

kidney cyst ( J:23047 , J:27506 )

• cysts are evident at birth and increase in number and size with age (J:23047)

• cysts are initially found in the inner cortical and medullary regions, by 4 days of age cysts have expanded to involve most areas of the kidney including the outer cortex, and by 7 days virtually the entire kidney is affected (J:23047)

liver/biliary system

dilated bile duct ( J:23047 )

enlarged gallbladder ( J:23047 )

• about 50% of homozygotes have an enlarged gallbladder

digestive/alimentary system

dilated pancreatic duct ( J:23047 )

• dilation of the pancreatic duct

growth/size/body

enlarged gallbladder ( J:23047 )

• about 50% of homozygotes have an enlarged gallbladder

kidney cyst ( J:23047 , J:27506 )

• cysts are evident at birth and increase in number and size with age (J:23047)

• cysts are initially found in the inner cortical and medullary regions, by 4 days of age cysts have expanded to involve most areas of the kidney including the outer cortex, and by 7 days virtually the entire kidney is affected (J:23047)

decreased body size ( J:27506 )

distended abdomen ( J:23047 )

• seen at 3 to 10 days of age

endocrine/exocrine glands

dilated pancreatic duct ( J:23047 )

• dilation of the pancreatic duct

dilated bile duct ( J:23047 )

enlarged gallbladder ( J:23047 )

• about 50% of homozygotes have an enlarged gallbladder

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
autosomal recessive polycystic kidney disease		DOID:0110861	OMIM:263200	J:23047

Genotype
MGI:3582953

ht3

• Allelic Composition: Bicc1^jcpk/Bicc1⁺
• Genetic Background: involves: 101 * C3H * T STOCK

renal/urinary system

kidney cyst ( J:23047 )

• 4 out of 17 heterozygotes between 10 and 15 months of age display cyctic abnormalities in the glomeruli

growth/size/body

kidney cyst ( J:23047 )

• 4 out of 17 heterozygotes between 10 and 15 months of age display cyctic abnormalities in the glomeruli

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
autosomal dominant polycystic kidney disease		DOID:898		J:23047

Genotype
MGI:3582934

ht4

• Allelic Composition: Bicc1^jcpk/Bicc1^jcpk-bpk
• Genetic Background: involves: 101 * BALB/c * C3H * C57BL/6J * T STOCK

renal/urinary system

kidney cyst ( J:36348 )

• small opalescent cysts seen at 11 to 17 days of age

• cysts are radially arrayed, a pattern typical of cysts in the collecting ducts and cystic dilation of Bowman's space involving about 17% of glomeruli, similar to Bicc1^jcpk-bpk homozygotes

enlarged kidney ( J:36348 )

liver/biliary system

abnormal bile duct morphology ( J:36348 )

• tortuous ducts are generally lined with hyperplastic epithelium

dilated gallbladder ( J:36348 )

• gall bladder dilation is common

abnormal portal lobule morphology ( J:36348 )

• expanded portal tracts with multiple irregularly shaped and variably dilated bile ducts

abnormal portal triad morphology ( J:36348 )

• the portal triad is expanded by hematopoietic cells and immature stromal cells

pale liver ( J:36348 )

• liver parenchyma is pale

growth/size/body

kidney cyst ( J:36348 )

• small opalescent cysts seen at 11 to 17 days of age

• cysts are radially arrayed, a pattern typical of cysts in the collecting ducts and cystic dilation of Bowman's space involving about 17% of glomeruli, similar to Bicc1^jcpk-bpk homozygotes

distended abdomen ( J:36348 )

• visible by 7 to 10 days of age

enlarged kidney ( J:36348 )

endocrine/exocrine glands

abnormal bile duct morphology ( J:36348 )

• tortuous ducts are generally lined with hyperplastic epithelium

dilated gallbladder ( J:36348 )

• gall bladder dilation is common

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
autosomal recessive polycystic kidney disease		DOID:0110861	OMIM:263200	J:36348

Genotype
MGI:3583140

cx5

• Allelic Composition: Bicc1^jcpk/T(2;10)67Gso
• Genetic Background: involves: 101 * C3H * C3H/Rl * C57BL/6 * C57BL/RlGso * SEC/RlGso

renal/urinary system

polycystic kidney ( J:75799 )

• polycystic kidneys seen by 10 days of age

enlarged kidney ( J:75799 )

growth/size/body

polycystic kidney ( J:75799 )

• polycystic kidneys seen by 10 days of age

distended abdomen ( J:75799 )

enlarged kidney ( J:75799 )